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World J Radiol. Jun 28, 2014; 6(6): 252-260
Published online Jun 28, 2014. doi: 10.4329/wjr.v6.i6.252
Congenital hyperinsulinism: Role of fluorine-18L-3, 4 hydroxyphenylalanine positron emission tomography scanning
Jaya Sujatha Gopal-Kothandapani, Khalid Hussain
Jaya Sujatha Gopal-Kothandapani, Department of Paediatric Endocrinology, Royal Manchester Children’s Hospital, Manchester M13 9WL, United Kingdom
Khalid Hussain, Department of Paediatric Endocrinology, Great Ormond Street Hospital for Children NHS Trust and the Institute of Child Health, University College London, London WC1N 1EH, United Kingdom
Khalid Hussain, Developmental Endocrinology Research Group, Molecular Genetics Unit, Institute of Child Health, University College London, London WC1N 1EH, United Kingdom
Author contributions: Gopal-Kothandapani JS drafted the article; and Hussain K reviewed it critically, revised and approved the final version
Correspondence to: Dr. Khalid Hussain, Reader in Paediatric Endocrinology, Developmental Endocrinology Research Group, Molecular Genetics Unit, Institute of Child Health, University College London, 30 Guilford Street, London WC1N 1EH, United Kingdom. khalid.hussain@ucl.ac.uk
Telephone: +44-20-79052128 Fax: +44-20-74046191
Received: February 11, 2014
Revised: March 19, 2014
Accepted: May 16, 2014
Published online: June 28, 2014
Processing time: 136 Days and 16.9 Hours
Abstract

Congenital hyperinsulinism (CHI) is a rare but complex heterogeneous disorder caused by unregulated secretion of insulin from the β-cells of the pancreas leading to severe hypoglycaemia and neuroglycopaenia. Swift diagnosis and institution of appropriate management is crucial to prevent or minimise adverse neurodevelopmental outcome in children with CHI. Histologically there are two major subtypes of CHI, diffuse and focal disease and the management approach will significantly differ depending on the type of the lesion. Patients with medically unresponsive diffuse disease require a near total pancreatectomy, which then leads on to the development of iatrogenic diabetes mellitus and pancreatic exocrine insufficiency. However patients with focal disease only require a limited pancreatectomy to remove only the focal lesion thus providing complete cure to the patient. Hence the preoperative differentiation of the histological subtypes of CHI becomes paramount in the management of CHI. Fluorine-18L-3, 4-hydroxyphenylalanine positron emission tomography (18F-DOPA-PET) is now the gold standard for pre-operative differentiation of focal from diffuse disease and localisation of the focal lesion. The aim of this review article is to give a clinical overview of CHI, then review the role of dopamine in β-cell physiology and finally discuss the role of 18F-DOPA-PET imaging in the management of CHI.

Keywords: Congenital hyperinsulinism; Fluorine-18L-3, 4-hydroxyphenylalanine positron emission tomography; Focal congenital hyperinsulinism; Diffuse congenital hyperinsulinism; Ectopic congenital hyperinsulinism; Standardized uptake value

Core tip: This manuscript describes how the advent of fluorine-18L-3, 4-hydroxyphenylalanine positron emission tomography (18F-DOPA-PET) scanning has revolutionised the management of patients with a very complex condition called congenital hyperinsulinism. 18F-DOPA-PET scanning allows the accurate pre-operative localisation of the focal lesion in these patients which can then be surgically removed allowing complete cure from the hypoglycaemia.